Limitations..........

I remember every detail of the day he was born. How it felt to hold him in my arms for the first time. The way he smelled. The first time he smiled. The first time he rolled over. The first laugh. The first word. Unforgettable and overwhelming blessings that I am so deeply thankful for.

SMA has a nice way of sucking the life out of you. I promised myself after he was diagnosed that I would NEVER focus on the things he couldn't do. We would adjust things however we possibly could and make him able, as well as focus on what he can do. No matter what. Little did I know how hard that promise would be to keep.

I remember how frustrating it was even just last summer trying to piece together some of the toddler phrases that he used. How I longed for his speech to improve and how I looked forward to be able to have understandable, real conversations with him. Well now that is exactly what I have. He is such a joy to talk to and pleasure to be around. But with everything good, it seems that there is a challenge to follow.

As hard as I try to stay positive and strong for him, sometimes I completely fail. I too am trying to figure this all out and often I find myself not handling particular situations as well as I would like to. Then I remember, this life is hard. This situation is hard. There are some things that can only be learned through experience. What I have been given to handle is always trying but I wouldn't trade it for the world.

Last night was one of those times. When I longed for Noah to be able to have conversations, what I didn't think about was his awareness of himself and other people. That has been exceptionally difficult.

Since it has warmed up the boys and I sit outside for about an hour or so as we wait for Lane to get home. It's nice to enjoy the fresh spring air after such a long winter. Typically every evening Noah asks to play with either trains or cars, and last night he wanted cars. So as I sat with him he told me he wanted to stand on the dirt. Dirt meaning 'in the flowerbed'. I looked at him puzzled and repeated what he said and his response was "yes mama, I want to stand on the dirt". Still confused I asked him if he was wanting me to bring out his stander. He became very frustrated and yelled "no stander. I want my feet in the dirt and I want to stand".  I have no idea how long it took me to get words out of my mouth in response. I was totally speechless and in shock. How are you supposed to respond to something like that? I've dreaded this day for a while. He is now beginning to make observations and say things that completely baffle me. It was so heartbreaking. He went on and on begging me to stand. Something so simple that I myself take for granted....to be able to stand.

These are the challenges that I dread and fear that if I handle them incorrectly, I may damage him. I said the exact thing that I promised to never say, out of sheer lack of words...."you can't".  I Failed, miserably.

After crying with him, I told myself I am learning too. Next time, and I KNOW there will be one .....I will have myself better prepared.

Laryngomalacia....

Noah's first insignificant (we thought) medical diagnosis was laryngomalacia....

Laryngomalacia is a congenital softening of the tissues of the larynx (voice box) above the vocal cords. This is the most common cause of noisy breathing in infancy. The laryngeal structure is malformed and floppy, causing the tissues to fall over the airway opening and partially block it.
For most infants, laryngomalacia is not a serious condition — they have noisy breathing, but are able to eat and grow. For these infants, laryngomalacia will resolve without surgery by the time they are 18 to 20 months old. However, a small percentage of babies with laryngomalacia do struggle with breathing, eating and gaining weight. These symptoms require prompt attention.

Noah at 2 weeks old developed this awful noisy breathing. We took him to the pediatrician emergently all to be diagnosed with this and we were told that he would grow out of it....then it became normal and we didn't think of it again because it was fine....Right!???

About a month ago at Noah's routine pulmonary appointment, it was recommended that we see the ENT for Noah's laryngomalacia since he had not grown out of it......so much for it resolving on its own.

Around the time that all of Noah's other symptoms of SMA started, he also began choking and gagging severely on food etc. Hyper-focused on the other symptoms of SMA such as the non weight bearing and general floppiness the gagging wasn't as much of a concern because he was only 7 months old. We started to lay off on solid foods and he seemed to do somewhat better. A few months later, he was diagnosed with SMA and got a g-tube because of malnutrition. Slowly, he stopped eating altogether because he developed severe sensory issues to food. So now, fully g-tube dependent we have been working with an occupational therapist to get him interested in food and at least touching things he thinks are "yucky". There is only 1 food that we can get him to touch and taste/eat and enjoy without having a meltdown. French fries.......

2 weekends ago, we went out to breakfast at Denny's. Noah wanted a french fry and Lane was eating hash browns. I gave him a small piece of shredded hash brown only minutes later have him gagging and severely choking on it. More severely than he ever has.

So, a few days ago I began to research more on laryngomalacia and found that it can cause frequent apnea and choking/gagging....I think I have found the cause to part of our eating problems. He is scheduled to see an ENT on September the 23rd and I am praying that the doctor will be able to see the issue by doing a scope. If he does indeed have a more severe laryngomalacia, then surgery would be the next step. I would love to avoid another surgery, but if it would mean that he could be more comfortable and enjoy food then it would be worth it. My other fear is that SMA + congenital weak/floppy muscle in throat + normal progression of SMA sounds like disaster.

Please pray for him.......

August is SMA awareness month!

What does it mean to be aware??????? Per Webster being aware is: having or showing realization, perception, or knowledge. While most people in our lives know that Noah has SMA, but how many REALLY know what SMA is??? I'd be willing to bet not many. With that being said in honor of SMA awareness month, I'd like to take a moment and educate you, so that you may educate others. When Noah was first diagnosed, Lane asked the doctor what to expect in regards to Noah's future...her response was he will live a normal shortened life and he will just be in a wheelchair. That statement was so far beyond reality...while we face an entirely different spectrum of normal, it is far beyond just being in a wheelchair. SMA is a degenerative neuromuscular disorder that causes the muscles in the body to atrophy and waste away. With that being said SMA affects the ability to walk, talk, sit, stand, eat, breathe, and swallow. Something as simple as a cough, Noah is unable to do on his own. Therefore we have a machine that does it for him. It is degenerative and progressive...meaning without a cure Noah will continue to lose strength...in SMA there is no "building" muscle like so many think. 1 in 35 people are unknowingly carriers of the SMA gene. 1 in 6,000 babies are born with SMA. There is no treatment, and there is no cure.  

"SMA is caused by a missing or abnormal (mutated) gene known as survival motor neuron gene 1 (SMN1). In a healthy person, this gene produces a protein in the body called survival motor neuron (SMN) protein. In a person with mutated genes, this protein is absent or significantly decreased, and causes severe problems for motor neurons. Motor neurons are nerve cells in the spinal cord which send out nerve fibers to muscles throughout the body. Since SMN protein is critical to the survival and health of motor neurons, nerve cells may shrink and eventually die without this protein, resulting in muscle weakness. As a child with SMA grows, it is difficult for his/her weakened muscles to keep up with the demands of daily activities. The resulting weakness can also lead to bone and spine changes that may cause breathing problems and further loss of function."

While most aren't aware of what exactly SMA is, know that it's much more than a little boy being in a wheelchair. 

August is SMA awareness month, but for us SMA awareness is each and every day. Awareness is when we wake up in the morning and unhook our little guy's pulse oximeter, bipap and feeding pump. Awareness is when we move him to where he wants to be 100+ times a day because he can't. Awareness is picking up the toy he's dropped for the 10th time in 5 minutes because of the tremors. Awareness is when he looks at you and say's "momma, my legs are broken" Awareness is when I pick him up and hold his frail body. Awareness is when he says he wants to walk. Awareness is when I hold his hand and feel the tremors as they shake his tiny hand. For us, it's everyday, every thing. We need a cure.....In honor of Noah and August...it's only one month.....Educate yourself and spread the word. We need you!

Awareness is key to a cure!!!

Bad at updates :)

So I have been sitting here thinking at how much has happened since I last updated our blog.  Time is majorly lacking and I am exhausted all of the time....

Noah got RSV and pneumonia in January and I was hoping that by now he would be back to baseline but unfortunately he's not. SMA tends to progress a little more than normal during periods of illness. Most of the time he bounces back to where he was before he got sick but his hospitalization took a lot from him. I have kept hoping and praying his strength would come back but even his PT and OT noticed that he is weaker and has not regained what he had. He is still able to sit unsupported but very short periods of time. He falls over more often and he is not able to get himself back to a sitting position and is not able to hold his head up as well as he could before. I try to be strong but sometimes I just can't. The last few months have been pretty hard watching the progression and being totally helpless. It hurts, and cuts me down to the core....

In March, Noah finally got his very own power wheelchair. He loves it and I am so proud of him. He does amazing in it and I love that he has his independence.

The second week of June we drove to California for the annual Families of SMA conference at Disneyland. We left Wednesday night and got to Disneyland at 3am. It was rough on Noah. We were going to stop halfway and stay the night but the thought of unpacking all of his things...bipap, pulse ox, feeding pump, cough assist, just to pack them up in a few hours did not sound like fun, so we decided to just keep going. The first day consisted of sleep and registering for the conference. Noah's allergies had been giving him problems for the past few weeks and by the 2nd day of our trip we found out it wasn't just allergies. He began to run a fever and had junky lungs. I attended some of the workshops alone while Lane stayed in the room with the boys so that we didn't expose other kids to Noah's germs. He was sick basically the whole trip.

We went to Disneyland on Sunday and Monday and tried our best to enjoy it. The first day was hard because we didn't understand exactly the ropes of the wheelchair access stuff, but once we figured it out it was better. It was better, other than the fact that people are ignorant. Noah did great in his wheelchair for it being so very crowded and instead of people moving for him they stared at him. Some people laughed and said they wished their kids "had a cool cart" like his. Others asked if it was remote controlled and then we got the "what's wrong with him" remarks, To which I always reply...Nothing is WRONG with him..... Ignorance is bliss......truly. Instead of thinking we were a family on vacation just like everyone else, we were a spectacle. I even had a few people ask if they could take pictures because they had never seen anything like that before......ummm NO!

Last night I was sitting holding Noah in the playroom and Eli was pushing his walk behind walker all over the place..Noah was watching him, giggling and all the sudden he looked up at me and said "momma, I walkie on toes like Eli." I was completely heartbroken. I held it together, I stood up...wrapped my arm under his arms and around his chests and placed my other arm under his but and proceeded to help him walk. I moved his legs for him in a walking motion and he laughed and said "momma, I walkie". While I kept my composure while I was helping him, after I put him in bed I fell apart. It's so difficult. I believe harder on me than him. I just need to remember to show him and praise him on what he can do...I never want to be one to point out to him what he cannot do...I will always try to focus on what he can do...or figure out an alternative way to do things.....In my eyes he can do ANYTHING!!

Expectations.....

This post is meant to be insightful, not offensive.....

Expectations are a funny thing....just about as funny as people's perspectives.

Each and every morning minus the weekends I get up and get ready for work. Everyone still asleep when I leave. I make the 30 drive to work, put in an 8 hour day and then a 30 minute drive back home. In my life, 30 minutes is a lot of precious time. I usually get home from work around 5:30. Then a whole new marathon begins. I juggle homework, making Noah's food, feeding both kids and getting them bathed and in bed in 2 and a half hours by myself because Lane works evenings. Then back to homework again. In the midst of all of this I make Noah's appointments, deal with all of the SMA stuff and be his advocate, which is a full time job in itself.

Now I'm not complaining, simply giving insight to a day from my perspective.

This is where it all becomes funny. I have had multiple people say why don't you take a break, or why don't you ever have any time? Seriously?? When, How?  A breakdown of my day is never a good enough explanation which astounds me. I am one person....with a lot of daily tasks to complete. I know I am not perfect, I forget things and people A LOT! It is not intentional but my focus is the boys and school at the moment. It will be a miracle when I finish. At some point in my free time, Lane and I are going to put together a video of 24 hours of our life. Should be interesting.

So back to expectations, for some I am expected to do things on a dime. So for anyone who wonders why I haven't called, text or whatever it's not because I have forgotten about you or anything of that nature. It is simply that whenever I do get a free minute, I take a few breaths and get back to it. I love all of you that think and pray for us, we appreciate it very much. I am much better at receiving calls than making them. Hint, hint ;)   I am tired, stretched thin, overwhelmed and my emotions are a roller coaster. Thanks for your understanding. Please know I am trying........



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